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PubMed · 6644896

Primary bladder closure and bladder neck reconstruction in classical bladder exstrophy.

Abstract

The surgical results of 28 consecutive initial bladder closures and 25 consecutive initial bladder neck reconstructions performed for classical bladder exstrophy at our hospital between 1975 and 1982 are presented. Partial bladder prolapse occurred in 2 cases and complete wound dehiscence never occurred following the initial primary bladder closure. Urinary continence following bladder neck reconstruction was assessed from parental interviews. An excellent surgical result was defined either as achievement of a daytime dry interval for more than 3 hours or less than 1 incontinent episode per day. According to these parameters, an excellent surgical result was achieved in 86 and 80 per cent of children, respectively. In 21 children evaluated with excretory urograms between 1/2 and 6 years after bladder neck reconstruction 10 per cent of the renal units showed significant hydronephrosis and deterioration of function. The 2 patients who had upper tract deterioration were not followed postoperatively at our institution and the diagnosis of bladder outlet obstruction was delayed when excretory urograms were not obtained during the first postoperative year. This review of the surgical results following primary bladder closure and bladder neck reconstruction for classical bladder exstrophy demonstrates that secure abdominal wall closure and urinary continence can be achieved with minimal morbidity and with infrequent deterioration of renal function following staged functional bladder closure.

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BibTeXRIS

H Lepor, R D Jeffs. 1983. Primary bladder closure and bladder neck reconstruction in classical bladder exstrophy.. https://doi.org/10.1016/s0022-5347(17)51727-9

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MTHFR 677 TT genotype in a mother and her child with Down syndrome, atrioventricular canal and exstrophy of the bladder: implications of a mutual genetic risk factor?

Apart from Husmann and Vandersteen [in: Gearhart JP, Matthews R (eds) The Epispadias-Exstrophy Complex. Kluwer, New York, pp 199-206, 1999], we report only the second case of Down syndrome (DS) associated with exstrophy of the bladder (EB). Besides the appearance of DS, the newborn exhibited a complete atrioventricular canal (CAVC) and classical EB, including diastases of the symphysis, an epispadic penis and an open bladder plate. Despite current recommendations, the mother had not supplemented her intake of folic acid during the periconceptional period. In a comparable case, Al-Gazali et al. (Am J Med Genet 103:128-132, 2001) found the homozygous 677T allele of the methylenetetrahydrofolate (MTHFR) gene 677C-->T polymorphism in a mother and her child with DS and cervical meningomyelocele. They found that the mother, who also had not supplemented her folic acid intake, had a secondarily altered folate status with an increased homocysteine level, suggesting that the homozygous TT mutation in the MTHFR gene in both mother and her child had contributed to the presentation of DS and a neural tube defect. The combined clinical findings of the present case and the observations of Al-Gazali et al. led us to investigate the 677C-->T polymorphism in our mother-child pair. Likewise we found that mother and child were homozygous for the mutant 677T allele. Our findings support the suggestion of Al-Gazali et al. that the MTHFR 677TT could be a mutual genetic risk factor for the co-occurrence of trisomy 21 and midline defects, the risk of which may be reduced by periconceptional folic acid supplementation.

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